[Linear IgA bullous dermatosis: a review]

Abstract

Background: Linear IgA bullous dermatosis (LABD) is a rare auto-immune bullous disease occurring in adults or childhood.

Objective: Review of literature about physiopathology, triggering factors, clinical data and treatment of LABD.

Methods: Research on Medline and Embase database without any time limit until April 2010. Because of the lack of randomized therapeutic trials in LABD, retrospective series and case reports have been analyzed.

Results: LABD is due to IgA auto-antibodies typically directed against a proteolytic fragment of BP180 antigen, a 97 or 120kD protein, and/or other components of dermal-epidermal junction. The disease may be either idiopathic or triggered by several medication, most often vancomycin, but also other antibiotics, non-steroid anti-inflammatory, anti-hypertensive and anti-epileptic drugs… Clinically, eruption is typical in childhood with cluster and herpetiform arrangement of blisters and involvement of evocative anatomical sites. In adults, eruption is polymorphic, very atypical presentations are described. Diagnosis is confirmed by direct immunofluorescence which shows linear IgA deposition on the basement membrane zone. Immunoblot and immunoelectron microscopy are evocative in case of diagnosis hesitation. LABD may be associated with some inflammatory bowel disorders. There is no increased risk of cancer or lymphoma. For drug-induced LABD, withdrawal of the medication is followed by a quick healing of the lesions. Dapsone is quickly efficient in idiopathic LABD. Colchicine, sulfapyridine and systemic corticosteroids are used in case of intolerance or inefficiency of dapsone. Some authors emphasize the efficiency of first-intent antibiotics in LABD of childhood.

Conclusion: A triggering drug should be always suspected and stopped. Dapsone is the reference treatment in idiopathic cases of LABD.